Universal neonatal hearing screening

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Universal neonatal hearing screening
Other names: Early hearing detection and intervention (EHDI)
A newborn undergoing hearing screening.
PurposeEarly detection and intervention in deaf and hard-of-hearing infants[1]
Test ofHearing[1]
Based onOtoacoustic emission (OAE), automatic auditory brainstem response (AABR)[2]

Universal neonatal hearing screening (UNHS), also known as early hearing detection and intervention (EHDI), are programs that aim to identify, intervene, and follow-up babies and young children who are deaf or hard-of-hearing.[3][1] Without exposure to language in the first years of life, children will have difficult developing language skills.[4] Screening programs result in earlier diagnosis and improved language outcomes.[5]

It uses testing methods, usually otoacoustic emission (OAE) or automatic auditory brainstem response (AABR), to screen newborns.[2] These tests take about 10 to 20 minutes to perform and can be done after the first day of life in those who are at least 34 weeks gestational age.[5] Often the OAE is used initially, followed by the AABR in those in who there are concerns.[5] This may than be followed by diagnostic testing.[5] The goal is generally to screen babies before a month of age and put in place treatment by six months of age.[5] Screening is falsely positive in about 2% of cases.[5]

Universal neonatal hearing screening is mandatory in many developed countries.[2] In the United States universal screening of all newborns was recommended in 1993 by the NIH.[4] Previously the typical age at diagnosis was around 2 to 3 years old.[4][5] As such, children born with hearing loss historically performed worse educationally, with poorer language skills and social functioning.[4] When diagnosed early; however, outcomes are similar to those with normal hearing.[4] As of 2012, universal screening programs were uncommon in developing countries.[2]

Medical use

In order to be most effective in minimizing developmental delays and promoting communication, education and social development, timely and appropriate interventions need to follow the early identification of hearing loss. Interventions for children with permanent congenital hearing loss ranges from devices that amplify sound to devices that replace the function of a damaged inner ear to communication modalities including spoken language, sign language, and cued speech.

The choice of interventions depends on the degree and the cause of hearing loss, accessibility, affordability and family choice. For interventions to be effective, they should be appropriate, timely, family-centered and undertaken through a coordinated interdisciplinary approach, which includes access to specialists who have the professional qualifications and specialized knowledge and skills to support and promote optimal development outcomes.[6]

Key elements for ensuring the best outcomes for children with hearing loss may include:[7]

Early diagnosis and intervention for children with hearing loss can help them develop better communication skills. Children with hearing loss meeting the early identification and intervention guidelines were more likely to have enhanced vocabulary than children who did not meet EHDI 1-3-6 guidelines.[8] Projects have focused on improving EHDI process issues including improving LFU/LTD rates through at Women, Infants, and Children (WIC) certification screening visits.[9][10] and exploring technologies that can be incorporated in electrophysiological testing to facilitate hearing loss diagnosis in newborns, without the use of sedation.[11]

Investigators have reported on numerous topics including the screening and diagnostic evaluations of children with unilateral and mild bilateral hearing loss,[12] the impact of hearing loss and comorbidities,[13] and the long-term hearing loss risk of children born with cytomegalovirus.[14]

Rates of hearing loss

Hearing loss in neonates is the most common congenital birth defect and sensory disorder, and can be caused by a variety of reasons. Rates of significant permanent hearing loss in newborns os 1–2 per 1000 live births in the United States.[15][16] With screening, many forms of congenital hearing loss can be detected. Congenital hearing loss can be due to genetic causes, environmental exposures during pregnancy, or health complications shortly after birth.[17] In Europe and North America approximately 0.1% of children having a hearing loss of more than 40 decibels when health or education records are reviewed. Other international studies using different methods have reported higher estimates.[18] In the United States, studies have shown a wide range of estimates for the number of children with hearing loss depending upon the reported age range, type, degree, frequency, laterality and method of ascertainment (e.g. audiometric testing, parental report, record review). Audiometric data of adolescents aged 12 to 19 years obtained through the National Health and Nutrition Examination Survey (NHANES) identified 3% to 5% of adolescents with hearing loss of 25 decibels or more and 15% to 20% with hearing losses of greater than 15 decibels.[19]

Technique

Newborn hearing screening employs objective assessment methods, either with automated (ABR) or (OAE), or both for initial and/or rescreening procedures.[20]

Often a two-stage process occurs in the actual screening of the hearing. Children are screened with either otoacoustic emissions (OAE) or automated auditory brainstem response (AABR). Children passing the test receive no further assessment. Children who fail the initial screen are usually referred for a second screening assessment either with OAE's or AABR. Children failing this second assessment will usually be sent for diagnostic assessment of their hearing. There is some variation in procedure by region and country but most follow this basic principle.[2]

Screening personnel vary also, in some regions Audiologists are used, whereas technicians, nurses, or volunteers are used in other programs. In countries that have insufficient financial and human resources to implement hearing screening, community-based programs have used simple, behaviour-based questionnaires to identify infants with hearing loss but with limited success.[2]

Targeted screening

Targeted neonatal hearing screening describes the process by which only a specific subset of a population are screened (for instance those infants in the neonatal intensive care unit or with risk factors for hearing loss). Although the U.S. Joint Committee on Infant Hearing (JCIH) endorsed the goal of universal detection of infants with hearing loss in 1994, it modified and maintained a role for specific high risk factors described in their previous (1973, 1982 and 1990) position statements.[21]

Successes

Universal newborn hearing screening programs aim to have high coverage rates (participation) and many aim to screen babies by one month of age, aim to complete the diagnostic process for referred babies by three months of age, and aim to begin intervention services by six months of age.[22]

U.S. jurisdictional programs (states and territories) screened less than 3% of all newborns for hearing loss in the U.S. at the beginning of the 1990s.[23] Data from states and territories for the early years of EHDI (1999–2004) were collected using surveys conducted by the Directors of Speech and Hearing Programs in State Health and Welfare Agencies[24] and shared with the CDC EHDI Program.[25][26] Beginning in 2005, CDC obtained data through an Office of Management and Budget approved survey sent to EHDI programme directors. In 1999, 22 jurisdictions estimated that less than half of all infants (46.5%) were screened for hearing loss, steadily increasing to 80.1% in 2005 85.4% in 2007, and 98.0% by 2009.[27] The number of deaf and hard of hearing babies identified early in life in the U.S. has steadily increased from 855 in the year 2000, 2,634 in 2005 and most recently reported 6,337 in calendar year 2016.[28]

Challenges

In addition to meeting the "1-3-6" targets, one of the key challenges for newborn hearing screening programmes is to reduce 'loss to follow-up' (where a child does not return for the next stage of the process). The Joint Committee on Infant Hearing (US) has reported that this is a significant problem in state screening programmes in the United States and other jurisdictions. Measuring loss to follow-up is an important step in understanding and reducing it.[29]

Now that over 95% of U.S. infants are having their hearing screened, remaining challenges include ensuring timely diagnostic evaluation for those who do not pass the screening and enrollment in early intervention for those with diagnosed hearing loss. In 2005, >60% of infants who had not passed the final or most recent screening were lost-to-follow-up/lost to documentation (LFU/LTD).[30] Some of those infants may have received audiologic evaluations, but the results not reported to the EHDI programme (i.e., undocumented evaluation). By 2007, LFU/LTD among infants not passing the final or most recent screening had decreased to approximately 46% and to 35% in 2011. The LFU/LTD percent for diagnosis in 2016 was 25.4% (n = 16,522). The 2016 LFU/LTD percent for enrollment in early intervention was 19.6% (n = 1,239).[27]

Challenges to newborn hearing screening have existed for over three decades.[31] Newborn screening alone can miss postnatal, progressive or acquired hearing loss, there is poor identification of perinatal infections, and concerns over regulatory barriers and privacy continue to this day.[32] Many infants are lost to follow-up and many families face the challenge of navigating coordinated quality care through complex health to education systems involving multiple agencies.[33][34][35]

History

In 1956, Erik Wedenberg published one of the earliest articles describing examiner use of tuning forks, percussion sounds, pitch pipes, and cowbells to screen the hearing newborn infants. The author noted, "until recently it has not been considered possible to carry out reliable auditory tests until the child has attained the age of 6–7 years."[36]

In 1963, Marion Downs, affectionately referred to as the "mother of pediatric audiology", pioneered the first hospital based infant hearing screening programme in Denver, Colorado, using Behavioral Observation Audiometry (BOA). Several independent observers recorded eye-blink and/or startle responses after presentation of narrow-band (90 dB) and white noise (93 dB) stimulation. In her 1964 publication, the observers identified suspected hearing losses, although disagreements were significant for 26% of the infants.[31] In 1969, Marion led efforts for the formation of the Joint Committee on Infant Hearing (JCIH) to provide multi-disciplinary leadership and guidance in all areas of newborn and infant hearing issues.[37]

In their 1971 Position Statement, the JCIH determined the results of screening programs were inconsistent and misleading and, although recognizing the urgent need for early detection, recommended discontinuing routine behavioral hearing screening of newborn infants. In 1973 the Committee recommended that only infants with certain high risk factors have their hearing evaluated (five factors: family history; congenital perinatal infections; ear, nose or throat defects; low birthweight <1500 g; hyperbilirubinemia).[38]

In 1982, two additional risk factors were added (bacterial meningitis and birth asphyxia including low Apgar scores) and included the recommendation for BOA or physiologic screening of high-risk infants. At that time, the Committee did not recommend any specific device, although many programme were successfully utilizing automated ABR for newborn screening. Despite efforts and endorsements, the growth of high-risk screening in the United States was very slow.[39] In 1984, high-risk hearing registries only included an estimated 15 percent of the nation's newborn population and likely, that less than half of those infants had their hearing assessed. Other weaknesses identified that a restricted risk register will excludes approximately 50 percent of infants with hearing impairment.[40]

In 1989 Surgeon General C. Everett Koop, perhaps most remembered for his work related to abortion, tobacco, and AIDS, called for increased efforts to identify congenital hearing loss within the first year of life. In his words, "It's a tall order, yes, but if we all work together, I believe we can fill it."[41]

In 1990, the JCIH added three more risk factors (ototoxic medication, prolonged mechanical ventilation, syndromic stigmata) for a total of ten. Congressman James T. Walsh (R-NY) sponsored and introduced the first attempt at federal legislation with the Hearing Loss Testing Act of 1991[42] requiring the hearing testing of every child born in the United States at the time of birth and establishing uniform standards for such testing. Although the Committee on Energy and Commerce referred this legislation to subcommittee, Congressman Walsh continued to promote legislation throughout that decade as the co-founder and co-chair of the Congressional Hearing Health Caucus. In 1993, the National Institutes of Health Consensus statement initiated the concept of "1-3-6" as the monthly milestones for screening, diagnosis and intervention. Between 1993 and 1996, the National Center for Hearing Assessment and Management at Utah State University conducted the Rhode Island Hearing Assessment Project (RIHAP), which demonstrated the feasibility of using transient OAEs for universal screening.[43][44] In 1994, both the JCIH and the DSHPSHWA endorsed universal newborn hearing screening. In 1996, the US Preventive Services Task Force concluded that the evidence was insufficient to assess the balance of benefits and harms and assigned an "I Statement" grade for newborn hearing screening.[45]

All US states participate in Early Hearing Detection and Intervention (EHDI) programmes.[46] The birth of EHDI may be attributed to passage of the Children's Health Act of 2000 which included language from Walsh's proposed legislation and authorized the Secretary of the Department of Health and Human Services, acting through the Centers for Disease Control and Prevention and the Health Resources and Services Administration, to establish "statewide newborn and infant hearing screening evaluation and intervention programs and systems." Congress provided HRSA with the authority to support statewide services and the CDC with the authority to provide technical assistance for data management and applied research. In addition, the National Institute on Deafness and Other Communication Disorders at NIH provided the authority to continue a programme of research and development on the efficacy of new screening techniques and technology.[47]

The year 2000 Position Statement of the JCIH provided the Principles and Guidelines for Early Hearing Detection and Intervention Programs. In 2006, the HHS Secretary's Advisory Committee on Heritable Disorders in Newborns and Children (SACHDNC) included newborn hearing as one of the conditions to be included in their Recommended Uniform Screening Panel (RUSP).[23] With growing research evidence, in 2007 United States Preventive Services Task Force (USPSTF) recommended screening of hearing loss in all newborn infants with an assigned B grade. By 2010, 43 states enacted legislative statutes or written regulatory language related to universal newborn hearing screening.[48]

The electronic age of EHDI may have begun during the next decade. The Quality, Research, and Public Health (QRPH) Planning and Technical Committees of Integrating the Healthcare Enterprise published a series of EHDI technical documents.[49] These profiles promote the automated collection and communication exchange of EHDI data between clinical and public health information systems (results, demographics, care plans, quality measures). The U.S. National Library of Medicine maintains the Newborn Screening Coding and Terminology Guide[50] to promote and facilitate the use of electronic health data standards in recording and transmitting newborn screening test results. This includes EHDI standard vocabulary codes and terminologies, including Logical Observation Identifiers Names and Codes (LOINC), Systematized Nomenclature of Medicine — Clinical Terms (SNOMED CT). The National Quality Forum the Centers for Medicare & Medicaid Services, and the Joint Commission for hospital accreditation have endorsed and/or supported adoption of EHDI electronic quality measures.[51] EHDI-PALS (Pediatric Audiology Links to Services) provides web-based link to information, resources, and services for children with hearing loss.[52] In 2018, Health Level 7 (HL7) approved the Early Hearing Detection and Intervention (EHDI) Implementation Guide as a Normative Standard.[53]

Availability

Newborn hearing screening programmes exist in many countries, including the United States,[54] United Kingdom[55] New Zealand,[56] and the majority of countries making up the European Union.[57] In order to maximize language and communication competence, literacy development, and psychosocial well-being, the U.S. Joint Committee on Infant Hearing[37] endorses the goals that 1) all newborns should undergo hearing screening using physiologic measures prior to hospital discharge, but no later than one month of age 2) all infants whose do not pass screening should have appropriate audiologic diagnosis no later than three months of age and 3) all infants identified as deaf or hard of hearing in one or both ears should be referred to early targeted and appropriate intervention services as soon as possible after diagnosis, but no later than six months of age.

In 2014 to 2019 the International Newborn and Infant Hearing Screening (NIHS) Group asked via questionnaire for the status of the hearing screening in 196 states worldwide; data from 158 states were obtained: in 64 states there is no or less screening (38% of the world's population); in 41 states (38% of the world's population) >85% of the babies are screened. The mean living standard in these states was 10 times higher than in countries without screening. It could show how useful a hearing screening can be: average age at diagnosis of hearing disorders was 4.6 months for screened children and 34.9 months for non-screened children.[58]

References

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  29. ↑ American Academy Of Pediatrics, Joint Committee on Infant Hearing (October 2007). "Year 2007 position statement: Principles and guidelines for early hearing detection and intervention programs". Pediatrics. 120 (4): 898–921. doi:10.1542/peds.2007-2333. PMID 17908777. S2CID 79426871. Archived from the original on 2023-01-16. Retrieved 2023-01-14.
  30. ↑ Alam S, Gaffney M, Eichwald J (2014). "Improved newborn hearing screening follow-up results in more infants identified". Journal of Public Health Management and Practice. 20 (2): 220–3. doi:10.1097/PHH.0b013e31829d7b57. PMC 4470168. PMID 23803975.
  31. ↑ 31.0 31.1 Downs MP, Sterritt GM (1964). "Identification audiometry for neonates: a preliminary report". Journal of Auditory Research. Archived from the original on 2023-01-16. Retrieved 2023-01-14.
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  35. ↑ Shulman S, Besculides M, Saltzman A, Ireys H, White KR, Forsman I (August 2010). "Evaluation of the universal newborn hearing screening and intervention program". Pediatrics. 126 Suppl 1: S19–27. doi:10.1542/peds.2010-0354F. PMID 20679316. S2CID 9448008.
  36. ↑ Wedenberg E (1956). "Auditory tests on newborn infants". Acta Oto-Laryngologica. 46 (5): 446–61. doi:10.3109/00016485609120155. PMID 13361864.
  37. ↑ 37.0 37.1 "Joint Committee on Infant Hearing". Archived from the original on 2020-06-14. Retrieved 2019-03-01.
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  39. ↑ Mahoney TM, Eichwald JG (May 1987). "The ups and "downs" of high-risk hearing screening: The Utah statewide program". Seminars in Hearing. 8 (2): 155–163. doi:10.1055/s-0028-1091366.
  40. ↑ "Early identification of hearing impairment in infants and young children". NIH Consensus Statement. 11 (1): 1–24. 1993. PMID 8401641.
  41. ↑ Northern JL, Downs MP (2002). "Chapter 1: Hearing and Hearing Loss in Children". Hearing in children. Lippincott Williams & Wilkins. p. 4.
  42. ↑ Walsh, James T. (1991-05-06). "Actions – H.R.2089 – 102nd Congress (1991–1992): Hearing Loss Testing Act of 1991". www.congress.gov. Retrieved 2019-03-01.
  43. ↑ Maxon AB, White KR, Vohr BR, Behrens TR (April 1993). "Using transient evoked otoacoustic emissions for neonatal hearing screening". British Journal of Audiology. 27 (2): 149–53. doi:10.3109/03005369309077906. PMID 8220282.
  44. ↑ Vohr BR, Carty LM, Moore PE, Letourneau K (September 1998). "The Rhode Island Hearing Assessment Program: experience with statewide hearing screening (1993–1996)". The Journal of Pediatrics. 133 (3): 353–7. doi:10.1016/S0022-3476(98)70268-9. PMID 9738715.
  45. ↑ Nelson HD, Bougatsos C, Nygren P (Jul 2008). "Universal newborn hearing screening: systematic review to update the 2001 US Preventive Services Task Force Recommendation". Pediatrics. 122 (1): e266–e276. doi:10.1542/peds.2007-1422. PMID 18595973. S2CID 40563482.
  46. ↑ CDC (2017-11-14). "Information About EHDI State Programs". Centers for Disease Control and Prevention. Archived from the original on 2023-01-16. Retrieved 2019-03-01.
  47. ↑ 127569 (2016-07-26). "Early Hearing Detection & Intervention (EHDI)". American Academy of Otolaryngology–Head and Neck Surgery. Archived from the original on 2019-03-01. Retrieved 2019-03-01.{{cite web}}: CS1 maint: numeric names: authors list (link)
  48. ↑ White KR, Forsman I, Eichwald J, Munoz K (April 2010). "The evolution of early hearing detection and intervention programs in the United States". Seminars in Perinatology. 34 (2): 170–9. doi:10.1053/j.semperi.2009.12.009. PMID 20207267.
  49. ↑ "Profiles". IHE International. Archived from the original on 2023-01-16. Retrieved 2019-03-01.
  50. ↑ "Newborn Screening Coding and Terminology Guide". U.S. National Library of Medicine. Archived from the original on 2019-01-24. Retrieved 2019-03-01.
  51. ↑ "Electronic Clinical Quality Measure (eCQM) Standards Landscape" (PDF). HE Quality, Research and Public Health (QRPH) White Paper. IHE International, Inc. 6 February 2018. Archived (PDF) from the original on 16 January 2023. Retrieved 14 January 2023.
  52. ↑ "Early Hearing Detection & Intervention Pediatric Audiology Links to Services (EHDI-PALS)". Archived from the original on 2023-01-16. Retrieved 2019-03-01.
  53. ↑ "HL7 Version 2.6 Implementation Guide: Early Hearing Detection and Intervention (EHDI) Results Release 1". HL7 International. Archived from the original on 2023-01-16. Retrieved 2019-03-01.
  54. ↑ "EHDI Programs". U.S. Centers for Disease Control and Prevention. 2017-11-14. Archived from the original on 2023-01-16. Retrieved 2023-01-14.
  55. ↑ "NHS Newborn Hearing Screening Programme Home Page". UK National Health Service. Archived from the original on 2015-05-24. Retrieved 2023-01-14.
  56. ↑ "Universal Newborn Hearing Screening Programme". New Zealand National Screening Unit. Archived from the original on 2023-01-16. Retrieved 2023-01-14.
  57. ↑ Levêque, Alain; Tognola, Gabriella; Lagasse, Raphaël; Senterre, Christelle; Vos, Bénédicte (2016-06-01). "Organisation of newborn hearing screening programmes in the European Union: widely implemented, differently performed". European Journal of Public Health. 26 (3): 505–510. doi:10.1093/eurpub/ckw020. ISSN 1101-1262. PMID 27009037. Archived from the original on 2023-01-16. Retrieved 2023-01-14.
  58. ↑ Katrin Neumann, Harald A. Euler, Shelley Chadha, Karl R. White (2020). "A Survey on the Global Status of Newborn and Infant Hearing Screening". The Journal of Early Hearing Detection and Intervention. 2 (2): 63–84. doi:10.26077/a221-cc28. S2CID 229246375. Archived from the original on 2023-01-16. Retrieved 2020-02-03.{{cite journal}}: CS1 maint: multiple names: authors list (link)


External links

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